自体外周血干细胞移植治疗难治性Castleman病疗效观察并文献复习

Autologous Peripheral Blood Stem Cell Transplantation for Treatment of Refractory Castleman's Disease:Cases Report and Literature Review

目的观察自体外周血干细胞移植治疗难治性Castleman病（Castleman＇s disease，CD）的疗效。方法回顾性分析3例接受自体外周血干细胞移植治疗的难治性CD患者临床资料并复习相关文献。患者均为多中心性CD，中位年龄为42．7（31-57）岁，移植前淋巴结平均最大直径为30mm，预处理2例采用BEAM，1例采用司莫司汀＋依托泊甙＋马法兰。采集物CD34’细胞中位数为7．47（5．51-10．00）×10 6／kg，单个核细胞（MNC）中位数为3．16（2．48-4．01）×10 8／kg。结果患者接受移植前均为难治性CD。所有患者均移植成功，中性粒细胞绝对值（ANC）〉0．5×10 9／L的中位时间为移植后12（11-13）天，血小板〉20×10 9／L为移植后13（12-15）天，移植后症状体征明显好转，淋巴结均消失，平均消失时间为21天。3例患者均完全缓解，至最近随访时间均未复发，中位生存时间超过2年。而联合化疗后疾病进展的患者病死率达55．6％，CR率为16．7％，与自体干细胞移植相比存活率明显下降。结论自体外周血干细胞移植治疗疗效良好，可作为难治性CD的挽救治疗。

Objective To investigate whether high - dose chemotherapy with autologous peripheral blood stem cell transplantation （ auto - PBSCT） could improve the survival of patients with refractory Castleman＇s disease（ CD）. Methods The clinical data of three pa- tients with refractory CD admitted to our transplantation center were retrospectively analyzed and related literature were reviewed. All three patients were recognized as MCD. The median age was 42.7 （ 31 - 57） years. The average maximum diameter of swollen lymph node was 30mm before transplantation. Two patients were conditioned using BEAM protocol and another one received Semustine,Etoposide and Mel- phalan. The median number of CD34~ cell was 7.47（5.51 - 10.00） x 106/kg and that of MNC was 3. 16（2.48 -4.01） x 10S/kg. Results All the candidates who had failed to achieve CR or PR before transplantation were defined as refractory CD. The high - dose chemotherapy with auto - PBSCT procedure resulted in a total CR rate and a progressive and dramatic improvement of all features associat- ed with refractory CD, even with the complication disease. The median time to ANC 0.5 x 109/L was 12 （ 11 - 13 ） days, and to platelcts 20 x 109/L 13 （ 12 - 15 ） days. At the latest follow - up, there was no find of relapse. The median survival time （MST） of these patients was over 2 years. On the contrary, The mortality rate of patients who had undertook combined chemotherapy progressed to PD was up to 55.6% , accompanied by only 16.7% of CR. It was obvious that the combined chemotherapy had significantly less survival than auto - PBSCT. Conclusion High - dose chemotherapy with auto - PBSCT was an effective therapy for refractory CD.